Deep Brain Stimulation for the Treatment of Dopa-Responsive Dystonia: A Case Report and Literature Review

Background: The aim of this study was to observe the improvement in patients with dopa-responsive dystonia (DRD) after deep brain stimulation (DBS) of the globus pallidus internus (GPi).

Case description: A 27-year-old woman with a 22-year history of DRD underwent GPi DBS. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), the Hamilton Anxiety Scale (HAMA), and the Hamilton Depression Scale (HAMD) were used to regularly assess changes in the patient's condition after DBS treatment.

Conclusions: After 6 months of GPi DBS treatment, the total BFMDRS score of the patient was 5, a 94.28% improvement over the preoperative baseline score. In addition, the MMSE, MOCA, HAMA, and HAMD scores of the patient were 30, 30, 1, and 2, respectively. Intelligence and cognitive levels of the patient did not change significantly compared with the preoperative baseline level, whereas anxiety and depression status of the patient improved compared with the preoperative status. GPi DBS treatment can significantly improve the symptoms of systemic dystonia in patients with DRD, and to a certain extent improve the anxiety and depression status of these patients. Therefore, we conclude that GPi DBS is an alternative safe and effective treatment for patients with DRD.